RECOGNITION AND MANAGEMENT OF SYSTEMIC LUPUS-ERYTHEMATOSUS

Systemic lupus erythematosus (SLE) is an inflammatory systemic disease that causes organ damage by the deposition of autoantibodies and comp lement activating immune complexes or by vascular occlusion due to pro coagulant states associated with antiphospholipid antibodies. The vast majority of cases occur in women of childbearing age. SLE is diagnose d on the basis of its clinical manifestations and the demonstration of characteristic immunological phenomena, especially anti-nuclear antib odies. The prognosis in SLE has shown a distinct improvement over rece nt decades, the 5-year survival rate now approaching or exceeding 90%. The 15-year survival rate of 63 to 79%, on the other hand, underscore s the need for further advances in diagnosis and treatment of the dise ase. Management of the disease includes regular monitoring of disease activity, avoidance of predisposing factors and close supervision of t herapy. Drug therapy is guided by the activity and severity of the lea ding organ manifestations and ranges from nonsteroidal antirheumatic-d rugs to intensive treatment with cytotoxic agents, Corticosteroids rem ain irreplaceable for the control of acute flares. Antimalarials and a zathioprine are important long term drugs for treating mild or moderat e disease activity. Intravenous pulse cyclophosphamide is safer than o ther regimens and at least as effective as oral cyclophosphamide for s evere lupus nephritis. It is also effective in the treatment of centra l nervous disease and of other organ-threatening manifestations. Recen tly, an intensified protocol which included cyclophosphamide induced l ong term treatment-free remission in 60% of patients. The toxicity of cyclophosphamide is considerable, but can be ameliorated by various me asures. The value of several new immunosuppressants and other compound s remains to be determined.